title: Nodular regenerative hyperplasia related portal hypertension in a patient with hypogammaglobulinaemia.
abstract: Nodular regenerative hyperplasia (NRH) of liver is a relatively rare liver disorder, but a frequent cause of noncirrhotic portal hypertension. We present a lady with common variable immune deficiency who presented with upper gastrointestinal bleeding and deranged liver function tests but preserved synthetic function. Upper gastrointestinal endoscope showed bleeding gastric varices and non-bleeding oesophageal varices. Although her oesophageal varices were eradicated by repeated endoscopic band ligation, the gastric varices failed to resolve after repeated endoscopic histocryl injection and she eventually needed transjugular intrahepatic portosystemic shunt placement. Liver biopsy showed NRH. We review the association of hypogammaglobinaemia and NRH and discuss the appropriate management of portal hypertension in NRH.
authors.affiliation: Department of Gastroenterology, Glasgow Royal Infirmary, Glasgow G4 0SF, United Kingdom. docbarun@gmail.com
authors.forename: Barun Kumar
authors.initials: BK
authors.lastname: Lal
authors.forename: Adrian
authors.initials: A
authors.lastname: Stanley
mesh_terms: D000361:Agammaglobulinemia / Q000150:complications* / Q000175:diagnosis; D001706:Biopsy; D017074:Common Variable Immunodeficiency / Q000150:complications* / Q000175:diagnosis; D004659:Enbucrilate / Q000008:administration & dosage; D016099:Endoscopy, Gastrointestinal; D004932:Esophageal and Gastric Varices / Q000209:etiology; D005260:Female; D020518:Focal Nodular Hyperplasia / Q000175:diagnosis / Q000209:etiology*; D006471:Gastrointestinal Hemorrhage / Q000209:etiology; D016558:Hemostasis, Endoscopic; D006490:Hemostatics / Q000008:administration & dosage; D006801:Humans; D006975:Hypertension, Portal / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D008111:Liver Function Tests; D008875:Middle Aged; D019168:Portasystemic Shunt, Transjugular Intrahepatic; D012008:Recurrence; D016896:Treatment Outcome
chemical_list: D006490:Hemostatics; D004659:Enbucrilate
keywords: Gastro-oesophageal varices; Hypogammaglobilinaemia; Liver; Nodular regenerative hyperplasia; Portal hypertension